This gene encodes a cytosolic homodimeric zinc-binding enzyme that catalyzes the hydrolysis of acylated L-amino acids to L-amino acids and an acyl group and has been postulated to function in the catabolism and salvage of acylated amino acids. This gene is located on chromosome 3p21.1 a region reduced to homozygosity in small-cell lung cancer (SCLC) and its expression has been reported to be reduced or undetectable in SCLC cell lines and tumors. The amino acid sequence of human aminoacylase-1 is highly homologous to the porcine counterpart and this enzyme is the first member of a new family of zinc-binding enzymes. Mutations in this gene cause aminoacylase-1 deficiency a metabolic disorder characterized by central nervous system defects and increased urinary excretion of N-acetylated amino acids. Alternative splicing of this gene results in multiple transcript variants. Read-through transcription also exists between this gene and the upstream ABHD14A (abhydrolase domain containing 14A) gene as represented in GeneID:100526760. A related pseudogene has been identified on chromosome 18. [provided by RefSeq Nov 2010]